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T2 - Phase 2 - Systems Modules (II) > Renal - Neoplasia > Flashcards

Renal - Neoplasia Flashcards

1
Q

What is an angiomyolipoma?

A

A hamartoma

(comprised of blood vessels, smooth muscle. and adipose)

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2
Q

Angiomyolipomas are hamartomas associated with what condition?

A

Tuberous sclerosis

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3
Q

Angiomyolipomas are hamartomas comprised of what tissue type(s)?

A

Blood vessels;

smooth muscle;

adipose tissue

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4
Q

Angiomyolipomas are benign renal neoplasms found primarily in what organ(s)?

A

The kidneys

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5
Q

Renal cell carcinoma is a malignant epithelial tumor arising from what?

A

The kidney tubules

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6
Q

What is the classic triad of renal cell carcinoma presentation?

A

(1) Hematuria
(2) Palpable mass
(3) Flank pain

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7
Q

The classic triad of renal cell carcinoma presentation is hematuria, palpable mass, and flank pain. How often do they occur simultaneously?

A

Very rarely

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8
Q

What is the most common presenting symptom of a renal cell carcinoma?

A

Hematuria

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9
Q

Name a few paraneoplastic syndromes associated with renal cell carcinomas.

A

EPO secretion

Renin secretion

PTH-related-peptide secretion

ACTH secretion

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10
Q

True/False.

Commonly, renal cell carcinomas present as left-sided varicocele.

A

False.

Rarely, renal cell carcinomas present as left-sided varicocele.

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11
Q

Gross exam of a renal cell carcinoma reveals what?

A

A yellow mass

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12
Q

Microscopically, the most common subtype of renal cell carcinoma presents how?

A

Clear cytoplasm

(clear cell subtype)

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13
Q

Pathogenesis of renal cell carcinomas involves a loss of ______ (tumor suppressor), which leads to increased ______ (growth factor) and ______ (growth factor).

A

Pathogenesis of renal cell carcinomas involves a loss of VHL, which leads to increased IGF-1 and HIF-1-α.

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14
Q

HIF-1α increases secretion of which transcription factors?

A

VEGF and PDGF

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15
Q

Are renal cell carcinomas hereditary or sporadic?

A

Can be either!

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16
Q

Hereditary renal cell carcinomas are most commonly seen in what condition?

A

von Hippel-Lindau disease

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17
Q

von Hippel-Lindau disease typically presents in ______ (age group) and 50% present with ______ (laterality) renal cell carcinomas.

A

von Hippel-Lindau disease typically presents in young adults and 50% present with bilateral renal cell carcinomas.

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18
Q

Sporadic renal cell carcinomas classically arise in _________ (patient population) as a single tumor in the upper pole of the kidney.

A

Sporadic renal cell carcinomas classically arise in adult males as a single tumor in the upper pole of the kidney.

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19
Q

Sporadic renal cell carcinomas classically arise in adult males as a single tumor in what location?

A

The upper pole of the kidney

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20
Q

What is the major risk factor for sporadic renal cell carcinomas?

A

Cigarette smoke

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21
Q

Renal cell carcinoma TNM staging is unique in that the tumor analysis involves assessment of the tumor’s interaction with what?

A

The renal vein

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22
Q

In addition to their spread to the renal vein, renal cell carcinomas commonly spread to which lymph nodes?

A

The retroperitoneal lymph nodes

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23
Q

Wilms tumor is a malignant tumor of the ________ that is comprised of immature mesenchyme known as ________.

A

Wilms tumor is a malignant tumor of the kidney that is comprised of immature mesenchyme known as blastema.

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24
Q

True/False.

Wilms tumors are benign tumors comprised of blastema (mature renal mesenchyme), primitive glomeruli/tubules, and some stromal cells.

A

False.

Wilms tumors are malignant tumors comprised of blastema (immature renal mesenchyme), primitive glomeruli/tubules, and some stromal cells.

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25
A child presents with a large, unilateral flank mass. What is the most common renal tumor in children?
Wilms tumor
26
Describe the clinical presentation associated with Wilms tumor.
A child with a large, unilateral flank mass, hypertension, and hematuria.
27
Why do children with Wilms tumor present with hypertension?
Excess **renin** **secretion** by the tumor
28
Most cases (90%) of Wilms tumors are ___________ (etiology).
Most cases (90%) of Wilms tumors are **_sporadic_**.
29
Although most cases of WIlms tumor are sporadic, Wilms tumors are also associated with some syndromes. Name three.
**WAGR** syndrome **Denys-Drash** syndrome **Beckwith-Wiedemann** syndrome
30
Describe the features of WAGR syndrome.
**W**ilms tumors **A**niridia **G**enital abnormalities Motor **R**etardation
31
WAGR syndrome is associated with _deletions_ in what gene?
*WT1*
32
Describe the features of Denys-Drash syndrome.
(1) Wilms tumor (2) Progressive renal disease (3) Male pseudohermaphroditism
33
Denys-Drash syndrome is associated with _mutations_ in what gene?
*WT1*
34
Describe the features of Beckwith-Wiedemann syndrome.
(1) Wilms tumor (2) Neonatal hypoglycemia (3) Muscular hemihypertrophy (4) Organomegaly
35
Beckwith-Wiedemann syndrome is associated with _mutations_ in what gene?
*WT2* cluster | (particularly IGF-2)
36
What disorder is described below? ## Footnote *A 3-year-old child presents with aniridia, a large, left flank mass that does not cross the midline, genital abnormalities, and some lack of motor function.*
**WAGR** **syndrome** *(**W**ilms tumors* ***A**niridia* ***G**enital abnormalities* *Motor **R**etardation)*
37
What disorder is described below? ## Footnote *A 2-year-old child presents with a large, left flank mass that does not cross the midline, ambiguous external genitalia, and elevated serum BUN. Karyotyping reveals that the child is XY.*
**Denys-Drash syndrome** * ((1) Wilms tumor* * (2) Progressive renal disease* * (3) Male pseudohermaphroditism)*
38
What disorder is described below? ## Footnote *A newborn presents with umbilical hernia, hypoglycemia, overgrowth of the lower extremity musculature, and macrosomia. Two years later, the patient is found to have a large, unilateral right flank mass that does not cross the midline.*
**Beckwith-Wiedemann syndrome** * ((1) Wilms tumor* * (2) Neonatal hypoglycemia* * (3) Muscular hemihypertrophy* * (4) Organomegaly)*
39
Malignancies of the lower urinary tract are defined as malignancies arising in what locations?
Renal pelvis; ureter; bladder; urethra
40
Most malignancies of the lower urinary tract (squamous cell, urothelial, adenocarcinoma) occur in what organ?
The bladder
41
What is the most common type of malignancy of the lower urinary tract?
**Urothelial carcinoma** | (a.k.a. transitional cell carcinoma)
42
Urothelial carcinomas (transitional cell carcinomas) most commonly arise in what location?
The bladder
43
Urothelial carcinomas (transitional cell carcinomas) are most commonly seen in what patient population?
Older adults
44
What is the major presenting sign/symptom of urothelial (transitional cell) carcinomas?
Painless hematuria
45
What is the **major** **risk** **factor** for urothelial (transitional cell) carcinomas? Can you name any others?
**Cigarette smoke**; naphthylamine, azo dyes, long-term cyclophosphamide or phenacetin use
46
Urothelial (transitional cell) carcinomas are often \_\_\_\_\_\_focal and _______ (do/do not) recur.
Urothelial (transitional cell) carcinomas are often **_multi_**focal and **_do_** recur.
47
What are the two main pathways from which urothelial (transitional cell) carcinomas arise?
(1) **Flat** (early P53 mutations) (2) **Papillary**
48
Which pathway of urothelial (transitional cell) carcinoma is associated with early P53 mutations?
The **flat** pathway | (as opposed to papillary)
49
Describe the progression of the flat pathway of urothelial (transitional cell) carcinomas.
Develops as a high-grade, flat tumor; then invades
50
Describe the progression of the papillary pathway of urothelial (transitional cell) carcinomas.
Develops as a low-grade, papillary tumor; then progresses to a high-grade tumor; then invades
51
Which form of urothelial (transitional cell) carcinoma begins as a high-grade tumor, invading soon after?
The **flat** form
52
Which form of urothelial (transitional cell) carcinoma begins as a low-grade tumor and then progresses to a high-grade tumor before invading?
The **papillary** form
53
Which form of urothelial (transitional cell) carcinoma is associated with early P53 mutations?
The **flat** form
54
**True/False**. Urothelial (transitional cell) carcinomas are often multifocal, but they typically respond well to typical resection.
**False**. Urothelial (transitional cell) carcinomas are often multifocal, ***and*** ***they*** ***recur***.
55
Squamous cell carcinomas of the lower urinary tract typically involve which organ?
The bladder
56
What typically must occur before a squamous cell carcinoma can arise in the lower urinary tract?
**Squamous metaplasia** (normal bladder and urinary tract surface is lined by urothelium)
57
*Schistosoma haematobium* is associated with what malignancy?
Squamous cell carcinoma of the bladder
58
Squamous cell carcinomas of the bladder associated with *Schistosoma haematobium* are most commonly seen in what patient population?
Egyptian males
59
Squamous cell carcinoma of the bladder that arises in an older woman is associated with what risk factor?
Chronic cystitis
60
**True/False**. The major risk factor for squamous cell carcinoma of the lower urinary tract is cigarette use.
**False**. The major risk factor for ***urothelial*** (***transitional*** ***cell***) carcinoma of the lower urinary tract is cigarette use.
61
Long-standing nephrolithiasis is a risk factor for what malignancy of the lower urinary tract?
Squamous cell carcinoma
62
\_\_\_\_\_\_\_\_\_\_\_ of the lower urinary tract is a malignant proliferation of glandular tissue, most often arising in the bladder.
**_Adenocarcinoma_** of the lower urinary tract is a malignant proliferation of glandular tissue, most often arising in the bladder.
63
A glandular malignancy arising at the dome of the bladder is most likely to be arising from what?
The **urachal** **remnant** | (The malignancy is an adenocarcinoma.)
64
Name an inflammatory condition that increases the risk of adenocarcinoma of the bladder.
Cystitis glandularis
65
Name a congenital abnormality that increases the risk of adenocarcinoma of the bladder.
Extrophy
66
Describe the histology of clear cell renal cell carcinomas.
Clear cells (in an acinar growth pattern) with intricate “chickenwire” vasculature

T2 - Phase 2 - Systems Modules (II) (22 decks)

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